Atypical anorexia nervosa (AAN) is a restrictive eating disorder (ED) that describes individuals who may be normal weighted or overweight; many have a premorbid history of obesity. Pediatric care providers are trained to identify and provide best practices for youth with pediatric obesity; however, most pediatric care providers are not trained to assess and treat restrictive EDs which typically present in youth aged 10 and 14 years. Although individuals with AAN may appear to be within a 'healthy weight', many experience malnutrition, psychological symptoms, and severe physiological complications after weight loss. These individuals are presenting to pediatric services at an increasing rate and exhibit acute medical instability along with severe ED psychopathology. One complicating factor is youth with AAN may take longer to be identified by pediatric providers and may be reluctant to engage in treatment. Delayed treatment for AAN, along with all EDs often results in poorer treatment outcomes. A greater understanding of this complex illness is essential to inform medical decisions, such as labs, vitals, hospital admissions, and psychological therapy. Currently, there are no standardized guidelines for treating AAN in youths. This review is designed to present evidence-based treatment to inform and guide best treatment practices.